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Year : 2019  |  Volume : 8  |  Issue : 4  |  Page : 286-289

Recurrent choroid plexus carcinoma

Department of Medical Oncology, NIMS, Hyderabad, Telangana, India

Date of Submission18-Oct-2019
Date of Acceptance26-Oct-2019
Date of Web Publication16-Dec-2019

Correspondence Address:
Dr. Ravi C Ambalath
Medical Oncology, NIMS, Hyderabad, Telangana -500 082
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Source of Support: None, Conflict of Interest: None


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Plexus-chorioideuscarcinomataor malignant plexus papillomas are very rare malignancy; only few cases have been described in the literature. A 2.5-year-old girl, known case of choroid plexus carcinoma, presented with complaints of weakness of right upper limb and lower limb, decreased activity, altered sensorium and vomiting. After re-evaluation, she underwent re-excision of tumor and chemotherapy started.

Keywords: Chemotherapy, choroid plexus carcinoma, recurrence

How to cite this article:
Ambalath RC, Konatam ML, Bala S, Gundeti S. Recurrent choroid plexus carcinoma. J NTR Univ Health Sci 2019;8:286-9

How to cite this URL:
Ambalath RC, Konatam ML, Bala S, Gundeti S. Recurrent choroid plexus carcinoma. J NTR Univ Health Sci [serial online] 2019 [cited 2020 Jul 13];8:286-9. Available from: http://www.jdrntruhs.org/text.asp?2019/8/4/286/273127

  Background Top

Choroid plexus carcinoma (CPC) is an uncommon intracranial neoplasm with virulent course and poor prognosis. Approximately 80% of CPCs arise in children which constitute about 15-20% of choroid plexus tumors (CPT).[1] The most common location is the lateral ventricles. Literature on CPCs is scant because of its rarity. Most are case reports or small case series.[2] We report a case of choroid plexus carcinoma in a 2.5-year-old child.

  Case Summary Top

A 2.5-year-old girl child without any comorbidities and normal developmental milestones presented with history of generalized tonic-clonic seizures, headache and vomiting of sudden onset. Past history and family history was not significant. A contrast-enhanced computed tomography (CT) scan of the brain showed a large well-defined heterogeneous lesion with cystic and hyperdense solid component with calcification, suggestive of supratentorial PNET/atypical teratoid-rhabdoid tumour/choroid plexus papilloma [Figure 1]. She underwent left posterior temporal craniotomy and near total excision of the tumor at another hospital.
Figure 1: Preoperative computed tomography: Noncontrast computed tomography brain showing large well defined hyperdense mass lesion in occipital horn of left lateral ventricle causing dilatation of occipital horn

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The histopathology was suggestive of CPC. Immunohistochemistry for pan cytokeratin was strongly positive and glial fibrillary acidic protein (GFAP) was positive in occasional tumor cells. She did not receive any adjuvant therapy.

Three months later, she presented with vomiting, altered sensorium and weakness of right upper and lower limb. A CT scan showed a recurrence of the lesion. Magnetic resonance imaging of brain showed a large heterogeneous mass in the left fronto-temporo-parietal region with significant mass effect [Figure 2]. She underwent external ventricular drain placement, left temporo-parietal craniotomy and gross total excision of the tumor. Intraoperative findings revealed a greyish tumor which was soft, suckable and moderately vascular and arising from choroid plexus of lateral ventricle. Postoperative period was uneventful. Postoperative histopathology was suggestive of CPC WHO (World Health Organization) Grade III. Immunohistochemistry showed Cytokeratin, INI1 positivity and synaptophysin, GFAP and epithelial membrane antigen (EMA) were negative [Figure 3]. Postoperative CT scan revealed near total excision of tumor. She was started on chemotherapy with IEC (Ifosfamide, Etoposide. and Carboplatin) regimen.
Figure 2: (a) Magnetic resonance imaging showing lesion which is hypointense on T1. (b) Magnetic resonance imaging showing lesion which is hyperintense on FLAIR with edema in left parieto-occipital region

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Figure 3: (a) HPE: Lesion is arranged in papillary pattern in partly. There is loss of papillary pattern and arranged in sheets. There is focal infiltration of lesion into adjacent brain parenchyma. High power view is showing nuclear pleomorphism and hyperchromasia. Large areas of necrosis are seen. (b) Immunohistochemistry: PAN-cytokeratin and INI1-Positive; Glial Fibrillary Acidic Protein, SYNAPTOPHYSIN, Epithelial Membrane Antigen-Negative

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  Discussion Top

CPT are rare central nervous system neoplasms accounting for 1-4% of all pediatric brain tumors.[3],[4] They are of neuroectodermal origin and most of the cases occur in infants, young children and rarely in adults. Choroid plexus tumors are categorized based on histological criteria as choroid plexus papilloma (CPP, WHO Grade I), choroid plexus carcinoma (CPC, WHO Grade III), and an intermediate entity termed atypical plexus papilloma (APP, WHO Grade II).[5]

Carcinoma of choroid plexus usually will have a virulent course and carry a poor prognosis. CPCs account for 15-20% of choroid plexus tumors and 80% of these are found in children. The most common site in children was lateral ventricles, as seen in the present case. CPCs are rare in adults and when diagnosed, most common site was fourth ventricle. Literature on CPCs is scant and only seven previous series were found in the past 30 years.

Clinically, CPCs cause hydrocephalus and increased intracranial pressure leading to convulsions, headache and rarely hemiplegia. A study by Bleggi-Torres et al. reported 15 cases of CPC, in which hydrocephalus, intracranial hypertension and convulsions were seen in 62.5%, 25%, 12.5% patients respectively. Neuroradiological features are nonspecific for CPC.

Most of the time, the diagnosis is established at histopathology. Sometimes, the distinction between choroid plexus papilloma and CPC is not obvious even with histopathology and in these cases immunohistochemistry becomes important in establishing the diagnosis, as happened in our case. CPP have histologic features that are very similar to those of the normal choroid plexus. The histologic criteria for malignant tumors of the choroid plexus, that is, CPCs were first developed by Lewis in the 1960s refined by Russell and Rubinstein two decades later, and most recently modified by the WHO. The established criteria are as follows: (1) obvious invasion of adjacent neural tissue with the infiltrating cells on a stromal base; (2) loss of regular papillary architecture; and (3) evidence of increased mitotic activity, nuclear atypia, and necrosis. CPCs typically stain positive for cytokeratins and display variable expression of vimentin, S100, transthyretin, and glial fibrillary acidic protein (GFAP). Positivity for S100 and transthyretin is typically less than that seen in CPP. CPCs stain positive for GFAP in approximately 20% of tumors.

Surgery is generally considered to be the most effective treatment and the most important prognostic factor.[6],[7] The extent of surgical resection remains the most important factor in determining long-term survival in patients with CPC, but patients treated only with surgery have had a very poor outcome, disease progresses rapidly, and patients often die within one year. The 5-year survival rate is 58% after complete tumor resection versus 20% after partial resection.[6] Adjuvant treatment of CPC may therefore be important not only for patients with partially resected CPC but also for patients with completely resected CPC.[5],[8]

In some small series, an objective response to radiotherapy was reported, showing that CPC is a radiosensitive tumor. Several case reports had described children with long-term survival who received radiotherapy. Larger analyses showed that radiotherapy improves the survival outcome of patients with CPC for incompletely and completely resected tumors. However, the late neurological sequelae of radiotherapy given to children under 3 years of age exclude this modality, leaving only chemotherapy as an adjuvant treatment for most patients with CPC.[5]

Although this tumor is still associated with a poor prognosis, there has been a slight but significant increase in survival throughout the past decades. Dohrmann and Collias reported a 9-month median survival time in a review of 16 children operated on for CPC.[9],[10] In 1992, Packer et al. reported 11 patients with CPC with a 45% event-free survival rate and a median progression-free time of 48 months.[7] Menon et al. reported median survival of 58 months for CPCs who underwent gross total excision with adjuvant therapy and of 36 months who had a subtotal resection with adjuvant therapy.[8]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. The 2007 WHO classification of tumours of the central nervous system. Acta neuropathologica 2007;114:97-109.  Back to cited text no. 1
Bleggi-Torres LF, Urban LA, Antoniuk A, Carboni P, Ramina R, Gugelmin ES. Choroid plexus carcinoma: Report of 15 cases. Arq Neuropsiquiatr 2000;58:505-11.  Back to cited text no. 2
Duffner PK, Kun LE, Burger PC, Horowitz ME, Cohen ME, Sanford RA, et al. Postoperative chemotherapy and delayed radiation ininfants and very young children with choroid plexus carcinomas. The pediatric oncology group. Pediatr Neurosurg 1995;22:189-96.  Back to cited text no. 3
St Clair SK, Humphreys RP, Pillay PK, Hoffman HJ, Blaser SI, Becker LE. Current management of choroid plexus carcinoma in children. Pediatr Neurosurg 1991;17:225-33.  Back to cited text no. 4
Wrede B, Liu P, Wolff JE. Chemotherapy improves the survival of patients with choroid plexus carcinoma: A meta-analysis of individual cases with choroid plexus tumors. J Neurooncol 2007;85:345-51.  Back to cited text no. 5
Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989;25:327-35.  Back to cited text no. 6
Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, et al. Choroid plexus carcinoma of childhood. Cancer 1992;69:580-5.  Back to cited text no. 7
Menon G, Nair SN, Baldewa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: An institutional series of 25 patients. Neurol India 2010;58:429-35.  Back to cited text no. 8
[PUBMED]  [Full text]  
Dohrmann GJ, Collias JC. Choroid plexus carcinoma. J Neurosurg 1975;43:225-32.  Back to cited text no. 9
Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E. Choroid plexus carcinomas in childhood: Clinical features and prognostic factors. Neurosurgery 1998;42:470-5.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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